Multiple Allergies, Hives and Abdominal Pain – Think Mastocytosis

I have seen cases of severe Irritable Bowel, combined with allergies and hives, that require multiple medications to control and are disabled from symptoms. These cases are discarded by gastroenterologists yet might represent a systemic disease called Mastocytosis.

World J Gastroenterol. 2008 Dec 7;14(45):7005-7008.
Gastrointestinal manifestations of systemic mastocytosis.
Lee JK, Whittaker SJ, Enns RA, Zetler P.

full free article here 

Mast cells are the mediators of allergies. They also trigger migraines:

see link here

They are found in large numbers in irritable bowel and interstitial cystitis (bladder) see here

So how can one tell when it reaches a level called mastocystosis?

• history of pigmented hives called urticaria pigmentosa -get a biopsy if you can.

• hives that on biopsy show “increased numbers of dermal mast cells highlighted by c-kit (CD 117) immunoperoxidase staining consistent with that of dermal mastocytosis”
• widespread chronic pruritis (itching)
• facial flushing – “widespread, patchy, violaccous, and evanescent, lasting only a few minutes, often occurs postprandially [after eating], and in the majority of cases not associated with hemodynamic changes [i.e. not dizzy with]” . Can be “bright red, pruritic, and burning”

Abdominal symptoms include:

• abdominal pain  51%
• diarrhea 43%
• nausea or vomiting 28%
• heartburn occasionally

Abdominal pain – two types

• Epigastric dyspeptic pain is associated with ulcer disease and acid hypersecretion
• lower abdominal cramps

variable abdominal symptoms include:

• steatorrhea (fatty pale stools) 5%-67%
• Hepatomegaly (large liver) 41%-72%

also can get telangiectasia macularis eruptiva perstans:

itchy pink flat patch symmetrical on abdomen with visible blood vessels (telangiectasias)

They make the mention that “Unfortunately, patients lacking skin manifestations of SM have a delayed diagnosis even though they often have more aggressive disease, impaired liver function, ascites,
malabsorption, and splenomegaly”

This is not well known even by hematologists (had a case I sent to one – had to look it up in the book).

Comment – I have a potential case that had some cells on abdominal biopsy but unfortunately no special staining was done. Perhaps it would be prudent to send cases to article group at:

Scott J Whittaker, Staff Gastroenterologist, St. Paul’s Hospital,
Division of Gastroenterology, University of British Columbia,
Vancouver V6B1K3, Canada
Robert A Enns, Staff Gastroenterologist, St. Paul’s Hospital,
Division of Gastroenterology, University of British Columbia,
Vancouver V6B1K3, Canada
Peter Zetler, Staff Pathologist, St. Paul’s Hospital, Division of
Pathology, University of British Columbia, Vancouver V6B1K3
Canada

(key author was a medical resident in training)

Measurement of serume tryptase levels helped establish diagnosis.

Treatments include antihistamines;

One available in Canada but not US is called Ketotifen (Zaditor) is particularly effective. Mixed with an H2 Blocker Ranitidine (Zantac) can lower tryptase levels:

J Allergy Clin Immunol. 1997 Dec;100(6 Pt 2):S25-32.
Heterogeneity of mast cells in mastocytosis and inhibitory effect of ketotifen and ranitidine on indolent systemic mastocytosis.
Kurosawa M, Amano H, Kanbe N, Akimoto S, Takeuchi Y, Yamashita T, Hashimoto T, Kurimoto F, Miyachi Y.   abstract here